MKSAP: 24-year-old woman with sickle cell anemia

Test your medicine knowledge with the MKSAP challenge, in partnership with the American College of Physicians.

A 24-year-old woman undergoes routine evaluation. She is pregnant at 12 weeks’ gestation. Medical history is notable for homozygous sickle cell anemia (Hb SS). She has had multiple uncomplicated painful crises treated at home with hydration, nonopioid analgesia, and incentive spirometry. She requires hospital management for these episodes approximately twice per year. She has declined the use of hydroxyurea. Her only other medication is folic acid.

On physical examination, vital signs are normal. Mild scleral icterus is noted. A grade 2/6 early systolic flow murmur is heard at the cardiac base. The examination is otherwise normal. Laboratory results show a hemoglobin level of 7.5 g/dL (75 g/L).

Which of the following is the most appropriate management?

A: Erythrocyte transfusion to maintain hemoglobin level at 10 g/dL (100 g/L)
B: Erythropoiesis-stimulating agent
C: Exchange transfusion
D: No transfusion at this time

MKSAP Answer and Critique

The correct answer is D: No transfusion at this time.

The patient should not receive an erythrocyte transfusion at this time. Erythrocyte transfusion in sickle cell disease (SCD) is appropriate only for specific indications, including stroke, symptomatic anemia, acute chest syndrome (ACS), surgical interventions, secondary prevention of stroke or ACS, and, possibly, prevention of priapism, pulmonary hypertension, and nonhealing ulcers. Transfusion is not indicated for uncomplicated pregnancy, routine painful episodes, minor surgery not requiring anesthesia, or asymptomatic anemia. Erythrocyte exchange transfusion is indicated for acute ischemic stroke, ACS with significant hypoxia, and multiorgan failure/hepatopathy as well as in persons in whom simple transfusion would increase the hemoglobin level to greater than 10 g/dL (100 g/L). Chronic transfusion can lead to iron overload, alloimmunization, and an increased risk for a delayed hemolytic transfusion reaction. Erythrocytes used in transfusion should be leukoreduced, hemoglobin S negative, and phenotypically matched for the E, C, and K antigens as well as for any known alloantibodies. Hemoglobin targets should remain less than 10 g/dL (100 g/L) to avoid hyperviscosity.

In a randomized trial, transfusion reduced the risk of pain crisis in pregnant women with SCD but showed no clear improvement in maternal mortality, perinatal mortality, or severe maternal morbidity (pulmonary embolism, chronic heart failure, ACS). Transfusion should be provided based on symptoms of anemia and not hemoglobin levels.

Erythropoiesis-stimulating agents (ESAs) are used to treat anemia in conditions in which bone marrow stimulation of erythrocyte production is inadequate, such as chronic kidney disease. However, in SCD, erythropoietin levels are typically high to augment bone marrow erythrocyte production in response to chronic hemolysis. Therefore, ESAs are not indicated for treatment of the anemia associated with SCD.

Key Point

  • In patients with sickle cell disease, including pregnant patients, transfusion is not indicated for uncomplicated pregnancy, routine painful episodes, minor surgery not requiring anesthesia, or asymptomatic anemia.

This content is excerpted from MKSAP 17 with permission from the American College of Physicians (ACP). Use is restricted in the same manner as that defined in the MKSAP 16 Digital license agreement. This material should never be used as a substitute for clinical judgment and does not represent an official position of ACP. All content is licensed to on an “AS IS” basis without any warranty of any nature. The publisher, ACP, shall not be liable for any damage or loss of any kind arising out of or resulting from use of content, regardless of whether such liability is based in tort, contract or otherwise.

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